Epstein-Barr virus (EBV) occasionally infects T and NK cells and causes EBV-infected T/NKcell lymphoproliferative disease (LPD), which comprises chronic active EBV infection, EBVassociated hemophagocytic syndrome, mosquito allergy, hydroa vacciniforme, aggressive NKcell leukemia, and NK/T-cell lymphoma. The diagnosis is proven by the monoclonal proliferation of EBV-infected T or NK cells, which is a time-consuming and complicated method. T-cell monoclonality is helpful for the screening of EBV-infected T-cell LPD in patients with EBVgenome burden and is easily shown with T-cell-receptor rearrangement or the T-cell repertoire, whereas NK-cell monoclonality is difficult to prove due to its lacking such rearranged receptors. We investigated a set of killer immunoglobulin-like receptors (KIRs) and also CD94-NKG2 heterodimers on NK cells, namely the NK-cell repertoire. Skewed repertoires were seen in all patients with EBV-infected NK-cell LPD, but not in any patients with EBV-infected T-cell LPD and were restored only after successful treatment. The normal KIR repertoire is variable for each individual and it seems difficult to detect minimal residual EBV-infected lymphocytes. However, the NK-cell repertoire is feasible for identifying EBV-infected NK-cell LPD and evaluating the treatment effect. Am. J. Hematol. 81:576-581, 2006. V V C 2006 Wiley-Liss, Inc.
We present a case of high-grade osteosarcoma in the proximal humerus in an 8-year-old boy. Massive tumor expansion required a S12345B shoulder girdle resection according to the system of the Musculoskeletal Tumor Society. After wide resection, only a small portion of the distal humerus and none of the rotator cuff muscles would be spared. Because the humeral portion would be too short to support the stem and the soft tissue would be insufficient to cover prosthetic components, we designed a composite reconstruction using a frozen autograft and a pedicled muscle flap. At 2 years postoperatively, our patient exhibited good adaptation and had acquired fine dexterity of the upper limb. To our knowledge, this is the first report regarding a successful composite reconstruction after a S12345B shoulder girdle resection.
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